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International Journal of Behavioral Medicine

, Volume 12, Issue 3, pp 171–179 | Cite as

A brief review of the pathophysiology, associated pain, and psychosocial issues in sickle cell disease

  • Christopher L. Edwards
  • Mischca T. Scales
  • Charles Loughlin
  • Gary G. Bennett
  • Shani Harris-Peterson
  • Laura M. De Castro
  • Elaine Whitworth
  • Mary Abrams
  • Miriam Feliu
  • Stephanie Johnson
  • Mary Wood
  • Ojinga Harrison
  • Alvin Killough
Article

abstract

Sickle cell disease (SCD) is the most common genetic disorder of the blood. The disease produces significantly abnormal hemoglobin (Hgb) molecules in red blood cells (RBCs). The sickling of RBCs occurs when partially or totally deoxygenated Hgb molecules distort their normal disk shape, producing stiff, sticky, sickle-shaped cells that obstruct small blood vessels and produce vasoocclusion as well as the disruption of oxygen to body tissues. Because tissue damage can occur at multiple foci, patients with SCD are at risk for other medical complications including, but not limited to, delayed growth and sexual maturation; acute and chronic pulmonary dysfunction; stroke; aseptic necrosis of the hip, shoulders, or both; sickle cell retinopathy; dermal ulcers; and severe chronic pain. The chronicity of the illness combined with frequent hospitalizations for pain and other medical management can contribute significantly to impaired psychosocial functioning, altered intra- and interpersonal relationships, and reduced quality of life. Unlike previous qualitative reviews of SCD, this article describes the relevant clinical and research data on the relation between psychosocial functioning and SCD in adult and child populations. The authors discuss the significant role of psychosocial issues in the trajectory and management of the disease and conclude that understanding the pathophysiology of SCD without thoroughly understanding the equally important psychosocial influences is misunderstanding SCD.

Key words

sickle cell disease psychosocial functioning chronic pain pain 

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Copyright information

© International Society of Behavioral Medicine 2005

Authors and Affiliations

  • Christopher L. Edwards
    • 1
    • 2
    • 3
  • Mischca T. Scales
    • 4
  • Charles Loughlin
    • 4
  • Gary G. Bennett
    • 5
  • Shani Harris-Peterson
    • 6
  • Laura M. De Castro
    • 7
  • Elaine Whitworth
    • 7
  • Mary Abrams
    • 7
  • Miriam Feliu
    • 8
  • Stephanie Johnson
    • 8
  • Mary Wood
    • 9
  • Ojinga Harrison
    • 10
  • Alvin Killough
    • 11
  1. 1.Department of Psychiatry and Behavioral SciencesPain and Palliative Care CenterUSA
  2. 2.Duke University Medical CenterDurham
  3. 3.Department of PsychologyNorth Carolina Central UniversityUSA
  4. 4.Department of Psychiatry and Behavioral SciencesPain and Palliative Care Center, Duke University Medical CenterUSA
  5. 5.Harvard School of Public HealthUSA
  6. 6.Johns Hopkins Bloomberg School of Public HealthUSA
  7. 7.Department of Medicine,Division of HematologyDuke University Medical CenterUSA
  8. 8.Department of Psychiatry and Behavioral SciencesDuke University Medical Center, Pain and Palliative Care CenterUSA
  9. 9.Department of PsychologyNorth Carolina Central University, Pain and Palliative Care Center, Duke University Medical CenterUSA
  10. 10.Department of Psychiatry and Behavioral SciencesDuke University Medical CenterUSA
  11. 11.Department of Library and Information SciencesNorth Carolina Central UniversityUSA

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